FAMILIAL HYPERINSULINEMIC HYPOGLYCEMIA

Familial Hyperinsulinemic Hypoglycemia is a condition that causes individuals to have abnormally high levels of insulin, which is a hormone that helps control blood sugar levels. People with this condition have frequent episodes of low blood sugar (hypoglycemia). In infants and young children, these episodes are characterized by a lack of energy (lethargy), irritability, or difficulty feeding.

HOW IS FAMILIAL HYPERINSULINEMIC HYPOGLYCEMIA DIAGNOSED?

A general physician diagnoses the disease by the following tests:
1. Physical examination of the clinical features
2. Blood test from the blood sample drawn during hypoglycemia
3. Urine test

HOW IS FAMILIAL HYPERINSULINEMIC HYPOGLYCEMIA TREATED?

Initially hypoglycemia is treated by injecting glucagon intramuscularly or intravenously or dextrose can be infused. Hypoglycemia due to drug overdose or effect is supported with extra glucose until the drugs have been metabolized. The drug doses or combination often needs to be altered. Hypoglycemia due to a tumor of the pancreas or elsewhere is usually curable by surgical removal. Most of these tumors are benign. Hyperinsulinism due to diffuse overactivity of beta cells, such as in many of the forms of congenital hyperinsulinism, and more rarely in adults, can often be treated with diazoxide or a somatostatin analog called octreotide. Diazoxide is given by mouth, octreotide by injection or continuous subcutaneous pump infusion. When congenital hyperinsulinism is due to focal defects of the insulin-secretion mechanism, surgical removal of that part of the pancreas may cure the problem. In more severe cases of persistent congenital hyperinsulinism unresponsive to drugs, a near-total pancreatectomy may be needed to prevent continuing hypoglycemia. Even after pancreatectomy, continuous glucose may be needed in the form of gastric infusion of formula or dextrose.